Primary biventricular repair for interrupted aortic arch with left ventricular outflow tract obstruction and tricuspid valve regurgitation; report of a case

An interrupted aortic arch was diagnosed in a 10-day-old girl weighing 3.3 kg, as was perimembranous ventricular septal defect (VSD) and severe tricuspid valve regurgitation (TR). The subaortic diameter was 3.6 mm and the aortic valve (3.7 mm in diameter) was bicuspid. We chose definitive repair, modified Yasui procedure, because of severe TR and no straddling of mitral valve. In primary biventricular repair, we undertook extended aortic arch anastomosis. Left ventricular outflow tract reconstruction consisted of intracardiac rerouting from the VSD to the pulmonary artery by using expanded-polytetrafluoroethylene (ePTFE) and Damus-Kaye-Stansel (DKS) anastomosis. Right ventricular outflow tract reconstruction was performed by the Rastelli procedure with an ePTFE valved conduit. Moreover, we carried out semicircular annuloplasty for severe TR.     

Closure of ventricular septal defect of interrupted aortic arch with left ventricular outflow obstruction]

Leftward shift of the infundibular septum (IS) in interrupted aortic arch (IAA) with ventricular septal defect (VSD) often develops significant left ventricular outflow obstruction (LVOTO). Seven-day-old boy with 2.6 kg body weight underwent the two-stage operation for this anomaly. The aortic arch was interrupted between the left common carotid and the left subclavian artery. At the first stage, a 5 mm GORE-TEX graft was used to connect the interrupted arch, and pulmonary artery banding was performed. In closure of VSD at the second stage, IS was penetrated by stitches for the VSD patch to left ventricular outflow tract. IS with leftward shift could be pulled toward right ventricular side with patch fixation and LVOTO was prevented by this method.     

Apico-aortic conduits in children with severe left ventricular outflow tract obstruction

BACKGROUND: Infrequently, congenital heart defects are complicated by left ventricular outflow tract obstruction (LVOTO) not amenable to conventional reconstruction. Apico-aortic conduits provide a means of palliating such patients until definitive repair is possible. The purpose of this study was to review a single institution's current experience with apico-aortic conduits. METHODS: The medical records of pediatric patients receiving apico-aortic conduits were reviewed. Demographics, operative techniques, preoperative and postoperative physiologic variables, morbidity, mortality, and functional class were recorded. Off-pump and on-pump procedures were categorized for comparison. RESULTS: Ten cases of apico-aortic conduits for left ventricular outflow tract obstruction were identified. Indications included congenital aortic stenosis, aortic atresia, and subaortic stenosis. Six procedures were performed off-pump and four required median sternotomy with cardiopulmonary bypass as necessitated by concomitant procedures. There was one operative death. The remaining patients demonstrated hemodynamic improvements and are all alive to date. One patient required conduit valve replacement. All patients are in New York Heart Association classification I or II at the time of last follow-up. CONCLUSIONS: Apico-aortic conduits provide a safe and effective treatment alternative for select cases of left ventricular outflow tract obstruction. Off-pump techniques are feasible in the majority of cases. This valuable adjunct should be considered whenever conventional repair of left ventricular outflow tract obstruction is considered prohibitive.     

Primary reconstruction of interrupted aortic arch by total aortic outflow obstruction

Primary aortic arch reconstruction was undertaken in three neonates with interrupted aortic arch and ventricular septal defect. Total ascending aortic occlusion without cardiopulmonary bypass or profound hypothermia permitted, in each case, a rapid tension-free end-to-side descending-to-ascending aortic anastomosis without resulting neurologic or cardiac sequelae. This technique offers distinct advantages over previously described methods and should be considered whenever interrupted aortic arch is present with a ventricular septal defect.     

Cavo-pulmonary shunt for left ventricular outflow tract obstruction after senning

We report a case of Senning procedure in a 32-month-old girl with transposition of the great arteries and dynamic left ventricular outflow tract obstruction (LVOTO). After discontinuation of cardio-pulmonary bypass, the persisting, worsened LVOTO caused hemodynamical instability. A cavo-pulmonary shunt (CPS) was performed, with considerable subsequent improvement. We consider the use of CPS as a valuable option in patients with persisting, significant dynamic LVOTO after atrial switch.     

An unusual cause of left ventricular outflow tract obstruction after mitral valve repair

Left ventricular outflow tract (LVOT) obstruction caused by systolic anterior motion is a cause of failed mitral valve repair. Intraoperative transesophageal echocardiography has been very helpful in diagnosing problems with mitral valve repairs intraoperatively, allowing immediate correction. We report an unusual cause of LVOT obstruction attributed to prolapse of the annuloplasty ring into the LVOT. Intraoperative hemodynamics were normal, and the diagnosis would not have been made before leaving the operative suite without the transesophageal echocardiography.     

Left ventricular rhabdomyoma with severe left ventricular outflow tract obstruction: development of delayed hemiplegia after cardiopulmonary bypass

The incidence of cardiac tumors increased with the improvement of imaging techniques in infants. Rhabdomyomas are the most common tumors in this group of patients. We herein report a 40-day-old male patient with left ventricular rhabdomyoma. The tumor caused syncope attack and supraventricular tachycardia. An emergency operation was planned and the life-threatening lesion was excised via left ventriculotomy. The patient was extubated on postoperative sixth hour and discharged from hospital on the sixth day of the postoperative period without any problem. This successful operation encourages us not to hesitate to perform an operation in newborns with cardiac neoplasms causing hemodynamic instability.     

Late outcome after arterial switch operation for complete transposition of great arteries with left ventricular outflow tract obstruction

BACKGROUND: Long-term follow-up of patients who underwent arterial switch operation for complete transposition of great arteries with anatomic left ventricular outflow tract obstruction (LVOTO) has rarely been brought into the focus. METHODS: Of 299 patients who underwent an arterial switch operation between January 1991 and January 2001, 23 patients had anatomic LVOTO. Age ranged from 4 days to 18 years (median 90 days) and weight ranged from 2.6 to 35 kg (median 4.3 kg). Surgical management included arterial switch operation, closure of ventricular septal defect wherever indicated, and excision of LVOTO. RESULTS: Among patients with preoperative LVOTO there were 2 early deaths and 8 patients had mild neoaortic regurgitation at the time of discharge. Follow-up ranged from 8 months to 9 years (mean 60 +/- 12 months). In 4 patients who had mild neoaortic regurgitation at discharge, the regurgitation progressed to moderate or severe degree after a follow-up of 22 to 72 months. In 1 patient mild mitral regurgitation present at the time of discharge progressed to severe mitral regurgitation. This patient subsequently underwent double valve replacement. CONCLUSIONS: Presence of preoperative anatomical LVOTO in patients undergoing arterial switch operation predicts high incidence of postoperative neoaortic regurgitation.     

Successful repair of TGA with ventricular septal defect, the outflow tract stenosis of the left ventricle and coarctation of the aortic arch]

A 9-month-old boy after the coarctectomy at 5 months old was successfully repaired by Senning procedure with closure of ventricular septal defect and resection of the membranous septal aneurysm causing the outflow tract stenosis of the left ventricle. Retrospectively, this case might have been possible to do Jatene procedure, however, attempts to resect the left ventricular outflow tract stenosis often failed to relieve stenosis completely. Therefore, the intraatrial switch operation seems to be better than the arterial switch operation when there is a possibility to remain left ventricular outflow tract stenosis. True estimation for these cases must await more case studies.     

Ross procedure for complex left ventricular outflow tract obstruction

BACKGROUND: Complex left ventricular outflow tract (LVOT) obstruction in children continues to pose a significant therapeutic challenge to cardiac surgeons. The Ross procedure, in combination with resection of subaortic stenosis or a Konno type septal incision, is an important option for these difficult patients. METHODS: Recently two children aged 14 and 5 years with LVOT obstruction involving combined subaortic and valvar stenosis underwent surgical correction using the pulmonary autograft. Clinical presentation, operative technique, outcome and intermediate follow-up are detailed. RESULTS: One patient had resection of an isolated subaortic membrane in combination with a pulmonary autograft and the second a Ross Konno procedure. Postoperative hospital stays were without complication. Both patients were discharged at 5 days and have no significant obstruction nor semilunar valve insufficiency at 3 years' follow-up. CONCLUSIONS: Pulmonary autografts can be used in combination with resection of subaortic tissue or a septal incision for reconstruction of complex left ventricular outflow tract obstruction. This technique renders excellent short term valve function, relief of obstruction, avoids anticoagulation and provides potential for future growth.     

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.